Dear Smile E.

I’m having a hard time maintaining my weight. Everyone is always after me to eat more; but how can I eat more when the food doesn’t have taste to it, because I can’t smell? I think my biggest issue is that I can’t stomach eating because so much of it tastes what I imagine sawdust tasting like. What are your suggestions on making food more tasteful and maintaining weight?

Signed,

Not Hungry in Texas

Dear Not Hungry in Texas,

I can totally relate to what you are going through. Maintaining weight when you’re a respiratory patient is difficult enough when we are well, let alone when we are fighting an infection. I saw a dietitian a few years back, and she told me that on average pulmonary patients need to consume about twice the amount of calories that a healthy person does just to maintain their weight. Let me explain. The average healthy person needs to consume around 2,000 calories a day to maintain optimal health. This gives their body enough energy to preform daily functions like breathing, heartbeat, moving, digesting, and other basic bodily functions. When a person has a pulmonary condition like PCD, their body works extra hard to breathe, fight infections, and so one; this requires extra energy. She suggested that I strive for about 3,800 calories a day on average due to my height, my weight, my age, and a few other factors. She also suggested getting these extra calories from high fat foods and high concentrate proteins instead of sugars and carbohydrates. Sugars and carbohydrates actually break down fast and make breathing more difficult. Whereas high fat foods and high concentrate proteins take longer to break down, and the energy they give you will stick with you longer than the energy from sugar and carbohydrates will. The biggest thing with respiratory patients is they often tire out easily when fighting infections, so high fat foods and high concentrate proteins will help with energy. Another thing is chose snacks with high protein count that are prepared, prepackaged, or in liquid forms. There are a wide variety or protein drinks, snacks, juices, and jellos too. Personal note, avoid the gelatin ones; so, so gross. 🤢 At least I thought so...

As far as taste, it truly does stink not to be able to smell your food. A few tricks my spouse taught me were spice it up, make sure it has plenty of moisture, and mix up the textures on your plate. First the spice it up; I know it sounds crazy, but pay attention to what makes your nose start running and add those spices into your food. I notice that when my nose runs a bit, while trying to eat, that occasionally I will get a whiff of a bit of the smell of my food. Sometimes, not always though. Play around with your spices and see what might enhance the flavor for you. Second the moisture; certain foods like steak, chicken, pork when dry really isn’t that appetizing to me; kind of like your description of sawdust actually. However give me a juicy steak or chicken and I’m all there. Soups and things like that, add in extra broth to your plate or bowl; and don’t forget the spices here either. Also don’t forget the creams; like heavy cream and sour cream. Be sure to add these to your soups or broths, they have the added benefit of those extra calories too. That’s a win, win in my book. Third and last mix up the textures; when you can’t use your sense of smell while eating to experience that pleasing side of food, look for alternative stimulation while eating to make food more enjoyable. Different textures like creamy smooth items, rough course items, chewy items, crisp items, and so on; on the same plate can really be engaging to and will definitely add stimulation from your other senses. Another note, I don’t personally do this, but a few friends who can’t smell like colorful plates as well. If it looks inviting that can stimulate interest in your meals as well.

Please keep in mind that I’m not a registered dietitian or a licensed physician, so be sure consult your care team for an individualized care plan that is tailored to your needs. The calorie counts above were again, based for me personally taking into to account my complete situation. Your calorie requirements may be totally different based of your overall health picture.

Yours Truly,

Smile E. Turtle

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Dear Ask Smile E.,

I don’t feel welcomed in the PCD groups lately. I mean I’m glad they or their child doesn’t have PCD as bad as mine does, but why demonize others with PCD and make them feel like they are doing something wrong and that is why they or their child is so sick with PCD? Smile E. Can you please give me a list of more supportive groups for sicker PCD patients? Are there any out there? I feel alone and looked down on.

Signed,

Feeling Like a Failure

Dear Feeling Like a Failure,

First let me start by again thanking you for allowing me a few weeks to answer this, per our correspondence. I appreciate the extra time to ponder this question and get some feed back from a few of our resources.

In short, it appears that there is no separate Facebook group for PCD patients with more significant disease and disability; yet. I do have a list of PCD support groups that I can private message you; so you can check if you are in all the PCD groups that are currently known to be in existence. I can also try to personally connect you up with PCDers or PCD families that have a similar situation to your own. And the PCD Foundation has a wonderful lady, who is a personal friend, that helps to connect up families with similar PCD situations as well. Sometimes that personal connection and personal conversation can go a long way in supporting one another through this difficult journey. If any of this interests you, please message us on Facebook or at my email where you sent this question to.

On a personal level; I’m really saddened by the fact that you haven’t been able to find the support that you need. I think sometimes, when we are in support groups, that in trying to be cheerful and supportive with encouragement that we forget that there are real people out there with PCD that are really struggling to make it. Even as a PCD patient, sometimes I find myself only sharing the good and briefly glossing over the bad parts. It’s sometimes very hard to admit you are struggling. And sometimes it is very hard to face what another PCDer is going through, because you worry that you will eventually end up there as well. It is definitely very important to note that Primary Ciliary Dyskinesia has such a broad spectrum of severity that it can be hard to judge where one falls on that spectrum. Some PCD patients live relatively normal lives with relatively normal FEV1s (lung function), and then there are those with very significant disease who require lung transplantation by early adulthood. The NIH (National Institute of Health) estimates according to their research that most PCD adults can have relatively stable periods of lung function but ultimately those same adults will have a decline in their lung function as they age; and many adults have lung function in the transplant range. I definitely hope that your questions will spark conversation within the PCD community about how we can be more inclusive to everyone in our community. I really wish that I could give you a big old hug, over coffee or tea, and tell you that you are not a failure; but a virtual hug will have to suffice for now.

Yours Truly,

Smile E. Turtle

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Dear Smile E.,

My spouse has a very hectic medication and treatment schedule. So they get tired and choose not to do their treatments. Or sometimes they don’t want to be a “bother”… How hard should I push for them to do them? Or should I even say anything?

Signed,

Worrying For My Love

Dear Worrying For My Love,

To be perfectly honest I’ve been there; I am there some days. As a PCD patient myself sometimes it’s very easy to just say I’m done, I need a break. And some days that’s okay, and some days it is not okay. In our world where we have little or no control over this beast called PCD, it is kind of liberating to say, “Nope not today PCD, I’m done.” Other times PCD seems inconvenient and it just gets in the way of things we’d rather be doing. Then there are the days that we absolutely too tired to move, and the effort to get our treatments or whatever ready to do seems too insurmountable to even attempt them. Being able to have some say over if we do our treatments makes us feel in control of an uncontrollable situation. I’m not saying it’s right or it’s wrong, it is what it is.

There is no magic answer to your question. Yes a gentle reminder maybe helpful, but constant nagging will only make you both miserable. However there does come a time where you need to sit down and have a real conversation with each other. Share your fears of the future with your spouse because of their skipping of treatments. Ask your spouse what you can do to help them navigate their hectic schedule. It might be something simple as helping them set up their machine in the morning while they are showering or what not. Or maybe they need help remembering to actually take time to do the treatments. Sometimes we all get busy, PCD or not, and forget to do something; we’ve all forgotten to eat before during an extremely busy day. Sometimes as patients ourselves we forget how our decisions to skip our treatments can impact others. Open and honest communication is always a great first step in navigating the journey with PCD.

Yours Truly,

Smile E. Turtle

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 Dear Ask Smile E.,

I’m reading as much as I can find about PCD, since our diagnosis. I want to be prepared, for my child’s future. I have seen several references to PCD patients and the need for lung transplants. What do I need to do to prepare for this outcome? And why are there so few references to lung transplants in PCD? And why does this topic seem to be taboo amongst the PCD community?

Signed,

Covering Our Bases

Dear Covering Our Bases,

I totally understand the need to be prepared for the journey that lies ahead for you and your child. The need for double lung transplant in PCD can be a sticky topic for many reasons that aren’t always clear. The road to transplant is often difficult, and also a road that we all hope to never go down. However lung transplants in PCD are very real. The actual statistics surrounding lung transplants in PCD patients are in my opinion skewed, mainly due to underreporting and or the lung transplant patient being misdiagnosed with something else; when in fact they actually have PCD. As PCD research has taken off in the past decade, so has PCD awareness. This awareness is helping to diagnosis the over twenty five thousand people that PCD Researchers believe are in the United States alone, who actually have PCD, but have been misdiagnosed into another disease/ disorder. This misdiagnosis means that there could be post-lung transplant patients out there who actually have PCD. Their new lungs do not have it any longer, but the rest of their body does. So let me explain.

The need for a PCD patient to have a lung transplant actually stems from the PCD patient having advanced stage bronchiectasis. So with PCD, basically the body’s natural filter for filtering out germs, bacteria, and debris is broken. That mans anything that a PCD patient breathes in, stays in. Normal clearance of bacteria, debris, and germs in a person without PCD is approximately one day or less. In a person who has PCD the same debris, bacteria, and germs take approximately seven days or more to be cleared. That is an extraordinarily longtime to clear debris, bacteria, and germs. It is also why PCD patients get sick from bacteria and uncommon bacteria that all around us in the soil, the water, and the air that would not normally make a human sick. The byproduct of PCD is bronchiectasis, which is the abnormal widening of airways that become scarred and the airway walls become thick from repeated chest infections seen in PCD. Once the bronchiectasis is so bad that it interferes with the lungs ability to exchange gases, then a lung transplant would be the only thing to extend the patient’s life. 

Transplant isn’t a cure in PCD, it’s just the end stage for the lungs. After transplant the patient will still have PCD everywhere else. And believing it or not the transplant patient will still have PCD in the new donor lungs; but only temporarily. After successful transplant, the new donor lungs have to basically wake up and start working in their new body. This means the donor lungs’ cilia are sometimes in a paralytic state or just plain sluggish. It can take six months or more for the donor lungs and it’s parts to be fully awake and fully operational. Mucoclearance and infection control are critical at this point of recovery. After transplant PCD patients still have to worry about PCD and the side effects that go with PCD. Those side effects make it a very real possibility that the PCD patient’s new donor lungs could become infected with not only bacteria, but uncommon bacteria as well. So there are still nasal washes, ear issues, and a whole host of other things that still go on after transplant for a PCD patient. 

As far as preparing for the possibility of transplant, I would suggest that you not worry yourself too much. The current data does suggest that transplants are not that frequent in PCD. However remember that data may be skewed due to inconsistent data and minsdiagnosises of PCD. Hopefully once the PCD Patient Registry is up and running for a few years, and we get some real longterm data following actual PCD patients this whole mystery of lung transplants in PCD patients will be answered with firm numbers and statistics. Until then our scientific data will be limited to the few research papers that are published to date. But it is important to stress that there is such a wide range in PCD with regards to symptoms and severity of disease due to a whole host of other reasons, including but not limited to which PCD causing gene defect that a patient has, that trying to compare your child’s path with PCD to another’s path with PCD is virtually impossible. There really is no way to say one way or the other if your child will eventually need a transplant or not. Then there is the whole matter of will your child qualify for the transplant program at the time in which your child might actually need the transplant. These are the reasons I believe transplant isn’t a huge topic in PCD; because each person with PCD, their experience is so individualized and unpredictable. The transplant journey just adds another layer of difficulty to a PCDer’s journey. Also keep in mind while preparing for you and your child’s future with PCD, that technology, research, and our understanding of PCD is ever evolving; so there is hope for the future. On a side note, if you’d Iike; I can try to connect you up with a transplant family or two, if they are willing and you have some specific questions that you’d like answered. Just email me if that would be of interest to you.

Yours Truly,

Smile E. Turtle

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