Today Freya is 9 months old. 9 months old. I can’t even compute that. It means that we are 7 months post-diagnosis, and yet it seems like only yesterday that we were taking our baby girl to A&E with a high temp and a low appetite, completely unaware that from that day on our lives would change forever. I had my precious baby girl at home with me for 7 weeks before that fateful day. Just 7 weeks; it was no time at all. Then Kawasaki Disease stole into our lives, and stole away my maternity leave.
I have previously shared Freya’s journey, our journey, with her illness through a number of blogs, taken from a journal that I wrote during our 6 weeks in the hospital from 31st May to 9th July 2015. Before she became ill, I had spent most of those early weeks resting, making the most of being a mature mum who had no desire to rush into doing anything too much at all. I was going to allow myself to enjoy this one, and the housework could wait. So almost all of those 7 weeks, give or take a few visits from friends and relatives here and there, were spent cuddled up on the sofa with my little ‘Peanut’, and I watched movies or slept to recharge my batteries after what had been a pretty tiring delivery. On the 30th May 2015, when my parents came to visit, we went for a day out to Cannon Hall Farm, a local attraction where my older children could feed the animals, and blow away some cobwebs with some fresh Summer air. I was content, no, more than that, I was happy beyond what I could ever have imagined when I found myself unexpectedly pregnant again at 40.
The following day, Freya awoke as normal, but instead of guzzling down that first bottle of the day she laboured through it with a stuffed up nose, and after what seemed like an age of feeding she’d managed about an ounce of formula (I had stopped breastfeeding the week before). Sure, I thought it was strange, but not being a particularly over-anxious parent I put it down to her snuffly nose, and decided to keep an eye on her. She didn’t take the next feed either; I guess that was around 9am, and whilst my concern was peaked, I didn’t make any rash judgements about the situation. She was sleepy, but what 7-week old baby isn’t? That’s all they do! Feed and sleep, sleep and feed. Except at that moment, Freya wasn’t too fussed about the feeding part. I would say it was around 11:30 or 12 when I decided that I couldn’t let her sleep any longer without trying to get some milk in her, so I made up a bottle and went to rouse her from the bouncy chair she was sleeping in. She looked warm, and was hot to the touch, so I fetched the digital ear thermometer and took her temperature. 38.3ºC. She had a fever.
Again, this didn’t panic me particularly, and I sent my husband to the local Mothercare store to buy a medicine dispensing dummy (I’d had one with my son, and it is a great way to get medicine into a tiny baby). While he was gone, I fished out the Calpol (paracetamol) and began to read the label for the right dosage. “From 2 months” read the label. Hmmm, should I give it to her? Was it safe? She was only a week off 2 months, and surely they were over-cautious with these things? But I couldn’t risk it – she was too precious, and I would never have forgiven myself if anything had gone wrong. Both my mother and mother-in-law were home with me, and both agreed that if I was concerned, I shouldn’t mess about. I should take her to A&E (the ER, for my American readers) – it was a Sunday, you see, so our GP surgery was closed (a factor in our KD journey that later I would be grateful for). I opted to call the out of hours surgery number, and after explaining that Freya had a snuffly nose, high temp and no appetite, and that her breathing was becoming quite laboured (her tummy was moving up and down, as opposed to her chest), they commented that it was unusual for a baby as young as Freya to have a fever and directed us to take her to the local hospital right away.
The rest, as they say, is history. But I often get asked about Freya’s journey, how we knew, what her symptoms were, so I thought it would be useful to document our experience with KD in 2015.
Week 1 (31st May to 6th June 2015)
- Visit to local hospital A&E with low appetite, high temperature and laboured breathing. Lethargy was not noted, as she was a tiny baby who was naturally sleeping a lot.
- Transferred to Children’s Observation Unit for observation. Immediate concern was Group B Strep as I had tested positive during the pregnancy.
- Admitted to Children’s Ward for overnight observation and placed on IV antibiotics as a precaution (standard procedure for suspected meningitis, which can develop in babies who have contracted GBS from the mother).
- Following morning around 5am, Freya develops a rash on her torso, which quickly spreads to her limbs. Doctor is called who states that Freya’s body is shutting down and she is wheeled to the High Dependency Unit (HDU).
- Freya is treated with a number of fluid boluses (“a rapid infusion of intravenous fluid or medication that is usually administered to correct a life-threatening condition”, Wisegeek.org). I don’t know what they put into her body, I just know that they acted like her life depended on it, and I looked on, heartbroken and in shock. I was alone; I called my husband and told him I thought he should come to the hospital.
- Remains in HDU for 3 days. IV antibiotics (Amoxycillin and Cefotaxime) and fluid are administered via cannula sites, as well as regular antipyretics (paracetamol and ibuprofen). Due to the size of the tiny veins, the cannula are unstable and new sites have to be found daily, sometimes more often. Monitors attached by wires to Freya’s body show a very high heart rate, and low oxygen levels. Temperatures continue to spike despite medication to bring them down.
- Condition stabilises, and Freya is moved back to the Children’s Ward. Feeding is via an NG tube up her nose, and she has cannulae in her wrists and ankles. Eventually a cannula is inserted in her head as the other sites fail. Blood tests and throat swabs are not conclusive and do not test positively for any standard illnesses. Continue to suspect GBS and/or bacterial meningitis. CRP reaches over 300. 3rd antibiotic is introduced (Gentamicin). Lumbar puncture is needed to diagnose meningitis, but Freya is too sick to undergo the procedure.
- Day 5, nurse confirms nasal swabs test positive for Rhinovirus (the common cold). One consultant states that Rhinovirus could make a tiny baby very sick. Another says “Rhinovirus does not do this to a child”. Chest x-rays and cranial ultrasound are clear.
- Day 6, Freya undergoes lumbar puncture. Results show raised white blood cells, but not high enough to indicate bacterial meningitis. However, Freya has been on 3 antibiotics for 6 days and it is considered that the condition may already have been fought off
- Day 7, we are told that on a scale of 1-10, with 11 being dead, Freya is a 10.
Week 2 (7th to 13th June 2015)
- Day 8, consultant (one we haven’t seen before) says that he is not happy that a diagnosis has not been reached and that if it was his child he would expect answers. Advises he has arranged transfer to a nearby Children’s Hospital where they had access to more resources, including the ability to carry out an echocardiogram (heart scan). Freya has her first ride in an ambulance. That evening I point out Freya’s swollen feet to the on-duty Consultant. He requests an x-ray on her chest, head and stomach, and asks for her foot to be x-rayed for suspected injury at the cannula site (I believe this was pre-peel swelling and a symptom of KD).
- Day 9, chest, head, stomach and foot x-rays are all clear. Echocardiogram shows a slight murmur, but is clear otherwise. Advised a murmur is not unusual in a newborn baby and usually resolves as the heart develops.
- Immunology and Infectious Diseases run various tests on daily blood samples. Antibiotics are replaced with Meropenem due to differences in hospital protocols. Acyclovir is also introduced (known for treatment of the herpes virus).
- Day 10, low haemoglobin levels result in Freya needing a blood transfusion.
- Day 11, MRI scan is undertaken to look for clusters in the brain that would indicate GBS infection. Freya is still so sick, she sleeps through the MRI scan and does not require a general anaesthetic. MRI results are clear.
- Day 12, Freya is placed under general anaesthetic in theatre, and undergoes a bone marrow aspiration to test her cells for Leukaemia. Tests are negative. Two further lumbar punctures are undertaken, but unable to collect any spinal fluid. Prolific rash appears on Freya’s arms and legs. Immunology Consultant requests Rheumatology opinion. Rheumatologist believes it is likely to be an infection, but requests a follow up echocardiogram and further blood tests.
- Day 13, sent for follow up echo, Sonographer says that he is looking specifically for signs of Kawasaki Disease. This is the first time I had heard those words. Sonographer apologises for putting his foot in it. Rheumatologist arrives to inform us that they have reached a diagnosis. Freya has Kawasaki Disease, and her coronary arteries are dilated. She requires immediate treatment of Intravenous Immunoglobulin, a blood product containing antibodies from thousands of donors. It is administered intravenously over 12 hours. Great Ormond Street and Newcastle Children’s Hospital are consulted and Freya is given methylprednisolone (high dose IV steroids) and aspirin (20mg). Seen by Ophthalmology to look for any issues with her eyes, nothing of note identified. All antibiotics are stopped. Contact made with Cardiologist at Leeds General Infirmary who says he is “not excited” by the coronary dilation.
- Day 14, Freya receives the second half of her IVIG infusion (due to her size she had to have the dose over 2 days). Echo shows no change from previous day.
Week 3 (14th to 20th June 2015)
- Day 15, follow up echo shows still no change from previous two days. CRP remains elevated. Rash has gone completely, and temperature spikes have reduced in frequency.
- Day 16, further echo shows significant increase in coronary artery dilation. The word aneurysm is mentioned. All three coronary arteries (left, right and left anterior descending) are dilated to over 5 times the normal diameter for a baby of her age. A second dose of IVIG is required. Contact made with Leeds Cardiology who insist that Freya is transferred to their Cardiac Unit. IV steroids stopped, and oral steroids are prescribed.
- Day 17, second half of second IVIG dose is administered and Freya is transferred to Leeds. Echo is carried out by Cardiologist who concurs with Sheffield’s findings and identifies a leaking aortic valve. Freya is admitted to the Cardiac HDU. Leeds Rheumatologist discusses possibility of administering a drug called Infliximab as they believe the inflammation is still ongoing.
- Daily echo’s show no improvement, but no worsening either. Request made to NHS England to give Freya Infliximab, not widely used in the treatment of KD, and not permitted for use in babies under 3 months (at that hospital at least). Funding agreed based on Freya’s case; “a rare and severe presentation” of “persistently active Kawasaki Disease” (letter from NHS England granting funding). Warned of the risks; Infliximab switches off TNF-alpha, the protein that helps the immune system to fight cancer (although it should be noted that this has been found in patients receiving the medication over prolonged periods, and no evidence has yet been found of malignancy following a single dose). Cardiologist explains the risks associated with Freya’s condition; stenosis (narrowing of the arteries), clotting (causing potential heart attack) or rupture (unpreventable without cure).
- Day 19, Infliximab infusion is given with no adverse reaction. Freya is transferred back to Sheffield the following day
Weeks 4-6 (21st June to 8th July 2015)
- Begin weaning steroid dose with a view to ceasing after 3 weeks.
- Bloodwork shows improvements. No temperatures or rashes. CRP dropping to near-normal levels (13 on Day 21).
- Day 26, ambulance transfer to Leeds for cardiology follow-up. No change. Advised unlikely to see any improvement for at least a year.
- Allowed home for day release, days 28 and 29. Discharged on Day 30 with instruction to return for bloodwork in one week.
- Day 31, Freya’s nappies show blood in her stools. Return to Sheffield Children’s Hospital with gastrointestinal bleeding. Re-admitted to the ward. Gastroenterologist wants to stop aspirin, Cardiology refuse. Administered IV lansoprazole. Great Ormond Street confirm there can be a small window to cease the aspirin if necessary. Freya’s formula is switched to Neocate, a mild non-cow’s milk protein formula. Bleeding stops within 24 hours. Ultrasound shows abnormalities in the arteries in Freya’s bowel. Barium swallow test shows normal function. Diagnosis: cow’s milk protein allergy exacerbated by KD inflammation and medication. Steroids weaned at faster pace than originally planned.
- Day 37, follow up Cardiology appointment in Leeds shows a reduction in the dilation of the coronary arteries (our “Wow!” moment) and the leaking valve has corrected itself.
- Day 38, Freya is well, but kept in as a precaution. Lips are still cracked and sore, bleeding when she cries. Finally discharged on Day 39 after Barium Swallow test returns a normal result. All bloodwork within normal levels, with the exception of ESR which remains slightly elevated.
13th July – follow up Rheumatology appointment (Sheffield) shows ESR remains elevated, but declining. Cardiology follow up (Leeds) shows no change from previous echo. Aspirin dose is increased based on Freya’s weight gain (standard anti-platelet dose in KD patients is 5mg per kilo).
6th August – Day 68, follow up Cardiology appointment shows further reduction in the dilation of the coronaries, with the right coronary artery (RCA) normalised, and the LCA reduced to 3mm (still large for a baby, but not considered as severe).
10th August – Rheumatology Follow-up (Sheffield) – happy with progress, all bloodwork has returned to normal ranges
13th August – Ophthalmology Follow-up (Sheffield) – discharged
3rd September – Gastroenterology Follow-up (Sheffield) – happy with progress
7th September – Cardiology Follow-Up (Leeds) – no change. Care transferred to Doncaster.
16th October – Gastroenterology Follow-up (Sheffield) – happy with progress
4th November – Cardiology Follow-up (Doncaster). No change. Request transfer back to Leeds due to poor experience.
11th November – Gastroenterology Follow-up (Sheffield) – happy with progress
And there it is. The last 9 months of my life on maternity leave. Writing it down like this has highlighted to me why I feel so exhausted, and so cheated of the year I had planned to take off with my new daughter. As I type, tears have begun to run down my cheeks. I think that’s what upsets me the most. The unfairness of it all. When I gave birth to Freya, when she was finally here, I decided it was time to banish all that negativity and really live life. I was going to enjoy every second of my maternity leave with her. This was a chance I wouldn’t get again. I imagined picnics in parks in the Summer sunshine; instead I watched other mums with their babies through a hospital window. I imagined meeting new people as I introduced my baby to new experiences; instead I kept her away from other children because of a repressed immune system, and later not being able to immunise or protect against the potentially fatal consequences of illnesses like the flu or chicken pox.
Don’t get me wrong, I have made some wonderful memories along the way, and in between the hospital stays and medical appointments, the calendar is peppered with visits to the Brontë Parsonage Museum, and Manchester’s Christmas Markets. But where there are blanks on the calendar, there have been numerous phone calls battling the prescription of Freya’s aspirin, countless hours of research, letters to MPs…the list goes on. The fact is that Kawasaki Disease stole my daughter’s health, took away my happiness, challenged my beliefs as parent and protector, and robbed me of my maternity leave.
Things are starting to settle down in 2016. We are waiting for Freya’s next cardiology appointment where we can only hope for good news. A blessing to stop researching for answers I will never find came in the form of e-mail responses from two cardiology professors, and specialists in Kawasaki Disease, and I feel a sense of relief from that. Freya has an appointment with Immunology at the end of this month where I hope to get some closure on the immunisation issue and develop a plan to catch up on the vaccinations that Freya missed, plus those that might save her from a worse fate (rare or not!). Perhaps we might even be able to take a holiday when we know she is less likely to pick anything nasty up on an airplane. In March we see the Dieticians at Sheffield Children’s Hospital to discuss the introduction of dairy into Freya’s diet once she reaches one year old. And following that, if there are no adverse reactions to cow’s milk in her diet, we should have our final Gastroenterology appointment. I am not sure how long we will continue to see Rheumatology, but they mentioned 2 years at one point, so I guess we will wait and see. That will leave us with Cardiology, which is likely to feature in Freya’s life for the foreseeable future. They said “for life”.
I have 3 months left with my beautiful daughter, before my maternity leave comes to an end. Instead of being a time of transition into the parental role, it has been a rollercoaster that I have wanted to get off from the first day Freya showed signs of illness. I have enjoyed many precious moments, overshadowed by sadness and fear. But I have known true love, and I have fought with every inch of me to keep my daughter safe. I cannot imagine a life without her in it, and because she is here I have to be grateful, even if the past year has been hateful. I cannot even start to think about leaving her in April. Seems the challenges for me are not over yet.